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Autoantibodies associated with immune-mediated necrotizing myopathy: pathogenesis and clinical implication

Research Project

Project/Area Number 17K09784
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Neurology
Research InstitutionKeio University

Principal Investigator

Suzuki Shigeaki  慶應義塾大学, 医学部(信濃町), 准教授 (50276242)

Project Period (FY) 2017-04-01 – 2020-03-31
Project Status Completed (Fiscal Year 2019)
Budget Amount *help
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2019: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2018: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2017: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Keywords自己抗体 / 炎症性筋疾患 / 免疫介在性壊死性ミオパチー / 抗SRP抗体 / 抗HMGCR抗体 / 抗合成酵素症候群 / 抗ARS抗体 / SRP / HMGCR / アミノアシルtRNA合成酵素 / 皮膚筋炎 / 壊死性ミオパチー / 筋炎
Outline of Final Research Achievements

Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve skeletal muscle as well as many other organs. In addition to a histological diagnosis at muscle biopsy, the clinical phenotypes of inflammatory myopathies can be defined by various autoantibodies that are originally detected by RNA or protein immunoprecipitation.Immune-mediated necrotizing myopathy (IMNM), characterized by significant necrotic and regeneration muscle fibers with minimal or no inflammatory cell infiltration, is associated with the presence of autoantibodies. IMNM is now classified as a distinct category of inflammatory myopathies. We divided autoantibodies into three groups: those associated with IMNM, those against aminoacyl transfer RNA synthetase, and those associated with dermatomyositis. The screening of autoantibodies has clinical relevance for managing patients with inflammatory myopathies.

Academic Significance and Societal Importance of the Research Achievements

近年,新たな病型や自己抗体の登場があり,筋炎の疾患概念には大きな変遷があった.我々の研究グループの一連の成果により,自己抗体の重要性が証明され,実地の臨床現場でも必須の検査となった.IMNMの患者血清中に特異的な自己抗体である抗SRP抗体と抗HMGCR抗体が検出される.「筋炎の統合的診断研究」では最も頻度の高い病型であるIMNMにおいて40%で抗SRP抗体,25%で抗HMGCR抗体が検出された.両者が同一の患者血清中に存在することはなく,互いに独立した血清マーカーである.我々はこれらの自己抗体の測定系を樹立し,実臨床で使用されている.今後,筋炎における自己抗体の病態機序の解明が望まれる.

Report

(4 results)
  • 2019 Annual Research Report   Final Research Report ( PDF )
  • 2018 Research-status Report
  • 2017 Research-status Report
  • Research Products

    (16 results)

All 2019 2018 2017 Other

All Journal Article (7 results) (of which Int'l Joint Research: 2 results,  Peer Reviewed: 7 results,  Open Access: 5 results) Presentation (5 results) (of which Invited: 5 results) Remarks (2 results) Patent(Industrial Property Rights) (2 results)

  • [Journal Article] Absence of sarcoplasmic myxovirus resistance protein A (MxA) expression in antisynthetase syndrome in a cohort of 194 cases2019

    • Author(s)
      Inoue M.、Tanboon J.、Okubo M.、Theerawat K.、Saito Y.、Ogasawara M.、Indrawati L. A.、Uruha A.、Okiyama N.、Fujimoto M.、Suzuki S.、Nishino I.
    • Journal Title

      Neuropathology and Applied Neurobiology

      Volume: 45 Issue: 5 Pages: 523-524

    • DOI

      10.1111/nan.12551

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Concurrent positive anti-3-hydroxy-3-methylglutaryl-coenzyme a reductase antibody with reducing body myopathy: Possible double trouble2019

    • Author(s)
      Tanboon Jantima、Sanmaneechai Oranee、Charuvanij Sirirat、Sangruchi Tumtip、Galindo-Feria Angeles S.、Lundberg Ingrid E.、Ohnuki Yuko、Shiina Takashi、Suzuki Shigeaki、Nishino Ichizo
    • Journal Title

      Neuromuscular Disorders

      Volume: 29 Issue: 7 Pages: 543-548

    • DOI

      10.1016/j.nmd.2019.05.007

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Anti-RNP antibodies delineate a subgroup of myositis : a systematic retrospective study on 46 patients.2019

    • Author(s)
      Wesner N, Uruha A, Suzuki S, Mariampillai K, Granger B, Champtiaux N, Rigolet A, Schoindre Y, Lejeune S, Guillaume-Jugnot P, Vautier M, Hervier B, Simon A, Granier F, Gallay L, Nishino I, Benveniste O, Allenbach Y
    • Journal Title

      Autoimmune Rev

      Volume: 19 Issue: 3 Pages: 102465-102465

    • DOI

      10.1016/j.autrev.2020.102465

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Inflammatory myopathy associated with PD-1 inhibitors.2019

    • Author(s)
      Seki M, Uruha A, Ohnuki Y, Kamada S, Noda T, Onda A, Ohira M, Isami A, Hiramatsu S, Hibino M, Nakane S, Noda S, Yutani S, Hanazono A, Yaguchi H, Takao M, Shiina T, Katsuno M, Nakahara J, Matsubara S, Nishino I, Suzuki S.
    • Journal Title

      J Autoimmun

      Volume: in press Pages: 105-113

    • DOI

      10.1016/j.jaut.2019.03.005

    • Related Report
      2018 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Cytometric cell-based assays for anti-striational antibodies in myasthenia gravis with myositis and/or myocarditis2019

    • Author(s)
      Kufukihara K, Watanabe Y, Inagaki T, Takamatsu K, Nakane S, Nakahara J, Ando Y, Suzuki S.
    • Journal Title

      Sci Rep

      Volume: 9 Issue: 1 Pages: 5284-5284

    • DOI

      10.1038/s41598-019-41730-z

    • Related Report
      2018 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Skeletal Muscle Involvement in Antisynthetase Syndrome2017

    • Author(s)
      Noguchi Eri、Uruha Akinori、Suzuki Shigeaki、Hamanaka Kohei、Ohnuki Yuko、Tsugawa Jun、Watanabe Yurika、Nakahara Jin、Shiina Takashi、Suzuki Norihiro、Nishino Ichizo
    • Journal Title

      JAMA Neurology

      Volume: 74 Issue: 8 Pages: 992-992

    • DOI

      10.1001/jamaneurol.2017.0934

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Nivolumab-related myasthenia gravis with myositis and myocarditis in Japan2017

    • Author(s)
      Suzuki Shigeaki、Ishikawa Nobuhisa、Konoeda Fumie、Seki Nobuhiko、Fukushima Satoshi、Takahashi Kikuko、Uhara Hisashi、Hasegawa Yoshikazu、Inomata Shinichiro、Otani Yasushi、Yokota Kenji、Hirose Takashi、Tanaka Ryo、Suzuki Norihiro、Matsui Makoto
    • Journal Title

      Neurology

      Volume: 89 Issue: 11 Pages: 1127

    • DOI

      10.1212/wnl.0000000000004359

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Presentation] 免疫チェックポイント阻害薬と自己免疫性神経・筋疾患overview.2019

    • Author(s)
      鈴木重明
    • Organizer
      第60回日本神経学会学術大会
    • Related Report
      2019 Annual Research Report
    • Invited
  • [Presentation] 免疫介在性壊死性ミオパチー2019

    • Author(s)
      鈴木重明
    • Organizer
      第31回日本神経免疫学会学術集会
    • Related Report
      2019 Annual Research Report
    • Invited
  • [Presentation] 免疫チェックポイント阻害薬の副作用とその対応.2019

    • Author(s)
      鈴木重明
    • Organizer
      第37回日本神経治療学会学術集会
    • Related Report
      2019 Annual Research Report
    • Invited
  • [Presentation] 抗ARSミオパチー2018

    • Author(s)
      鈴木重明
    • Organizer
      第59回日本神経学会学術大会
    • Related Report
      2018 Research-status Report
    • Invited
  • [Presentation] 免疫チェックポイント阻害薬と神経・筋における有害事象2018

    • Author(s)
      鈴木重明
    • Organizer
      第16回日本臨床腫瘍学会学術集会
    • Related Report
      2018 Research-status Report
    • Invited
  • [Remarks] 慶應義塾大学医学部神経内科ホームページ

    • URL

      https://www.neurology.med.keio.ac.jp/staff/6-2/

    • Related Report
      2019 Annual Research Report
  • [Remarks] 慶應義塾大学医学部神経免疫ホームページ

    • Related Report
      2017 Research-status Report
  • [Patent(Industrial Property Rights)] 抗横紋筋抗体の測定方法および測定試薬2019

    • Inventor(s)
      鈴木重明
    • Industrial Property Rights Holder
      鈴木重明
    • Industrial Property Rights Type
      特許
    • Filing Date
      2019
    • Related Report
      2019 Annual Research Report
  • [Patent(Industrial Property Rights)] 抗横紋筋抗体の測定方法および測定試薬2017

    • Inventor(s)
      鈴木重明・稲垣貴之
    • Industrial Property Rights Holder
      鈴木重明・株式会社TKResearch
    • Industrial Property Rights Type
      特許
    • Filing Date
      2017
    • Related Report
      2018 Research-status Report

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Published: 2017-04-28   Modified: 2021-02-19  

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